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Updates in: Congenital Diaphragmatic Hernia - Abstract

Merrill Mchoney



Multiple teams are integrally involved in the management of the neonate with CDH. Standardised antenatal ultrasound scanning and lung measurements are used to aid prognostication, if uniformity in measuring and reporting are used. Antenatal MRI seems to have a specific role in providing additional data for selection of antenatal intervention, which would be best suited for prospective randomised studies to report on benefit and outcome.

Conventional ventilation with minimal pressures to achieve oxygenation and permissive hypercapnia are the mainstay of initial ventilation. Initial PaCO2 and oxygenation index on day 1 has been shown to predict outcome. HFOV is often used with clinical benefit alongside the other management strategies for PPHN, including iNO and cardiovascular support. Sildenafil infusion enhances iNO mediated vasodilatation, and has been shown to be associated with improved oxygenation and outcome. Other vasodilators (milrinone and prostaglandins) can be considered depending on clinical course. ECMO is reserved for patients with severe pulmonary or cardiac compromise refractory to other modalities.

No overarching clear cut indication of the best timing of surgery exist, other than the achievement of relative stability. Serial oxygenation index as well as other cardio-respiratoy parameters (blood pressure, ductal shunting, urine output and lactate levels) can be used to indicate stability, and timing. Although the thoracoscopic approach to CDH is used, the limits of 'intraoperative permissive hypercapnia’ in CDH have not been established, and needs investigating in this setting.

Long-term morbidity requires multiple specialist follow-up, as respiratory, cardiac, surgical, nutritional and feeding issues are present. Postoperative and discharge follow up is also multidisciplinary, and a one-stop clinic follow-up is becoming more popularly the gold-standard for outpatient follow up.

Keywords: congenital diaphragmatic hernia, antenatal, FETO, PPHN, surgery, ECMO, outcome, follow-up, multi-disciplinary


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